Recombinant人MT-ND1 protein (ab114460)

概述

描述

  • 性质Recombinant
  • 来源Wheat germ
  • 氨基酸序列
    • AccessionP03886
    • 种属Human
    • 序列MLTERKILGYIQLRKGPNVVGPYGLLQPFADAIKLFTKEPLKPATSTITL Y
    • 分子量31 kDa including tags
    • 氨基酸21 to 71

技术指标

Our Abpromise guarantee covers the use of ab114460 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • 应用

    Western blot

    SDS-PAGE

    ELISA

  • 形式Liquid
  • 补充说明Protein concentration is above or equal to 0.05 mg/ml.
    ab114460 is best used within three months from the date of receipt.
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.79% Tris HCl, 0.3% Glutathione

常规信息

  • 别名
    • Mitochondrially encoded NADH dehydrogenase 1
    • MT-ND1
    • MTND1
    • NAD1
    • NADH dehydrogenase subunit 1
    • NADH dehydrogenase subunit 1 (complex I)
    • NADH-ubiquinone oxidoreductase chain 1
    • NADH1
    • ND1
    • NU1M_HUMAN
    see all
  • 功能Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.
  • 疾病相关Defects in MT-ND1 are a cause of Leber hereditary optic neuropathy (LHON) [MIM:535000]. LHON is a maternally inherited disease resulting in acute or subacute loss of central vision, due to optic nerve dysfunction. Cardiac conduction defects and neurological defects have also been described in some patients. LHON results from primary mitochondrial DNA mutations affecting the respiratory chain complexes.
    Defects in MT-ND1 are a cause of mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes syndrome (MELAS) [MIM:540000]. MELAS is a genetically heterogenious disorder, characterized by episodic vomiting, seizures, and recurrent cerebral insults resembling strokes and causing hemiparesis, hemianopsia, or cortical blindness.
    Defects in MT-ND1 may be associated with susceptibility to Alzheimer disease mitochondrial (AD-MT) [MIM:502500]. Alzheimer disease is a neurodegenerative disorder characterized by progressive dementia, loss of cognitive abilities, and deposition of fibrillar amyloid proteins as intraneuronal neurofibrillary tangles, extracellular amyloid plaques and vascular amyloid deposits. The major constituent of these plaques is the neurotoxic amyloid-beta-APP 40-42 peptide (s), derived proteolytically from the transmembrane precursor protein APP by sequential secretase processing. The cytotoxic C-terminal fragments (CTFs) and the caspase-cleaved products such as C31 derived from APP, are also implicated in neuronal death.
    Defects in MT-ND1 may be associated with non-insulin-dependent diabetes mellitus (NIDDM).
  • 序列相似性Belongs to the complex I subunit 1 family.
  • 细胞定位Mitochondrion inner membrane.
  • Information by UniProt

Recombinant Human MT-ND1 protein 图像

  • ab114460 analysed on a 12.5% SDS-PAGE stained with Coomassie Blue.

Recombinant Human MT-ND1 protein (ab114460)参考文献

ab114460 has not yet been referenced specifically in any publications.

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